Primary sclerosing cholangitis is a common cause of chronic liver disease in patients with ulcerative colitis. It is an autoimmune disorder and patients usually present with jaundice, fever, abdominal pain and elevated alkaline phosphatase. Endoscopic retrograde cholangiopancreatography is the best diagnostic tool to confirm the diagnosis of primary sclerosing cholangitis. Multiple strictures and dilations of the biliary system is usually observed. Symptomatic treatment, immunosuppression and ursodeoxycholic acid are used in early stage PSC. In later stages, liver transplantation might be the only option available to alter prognosis and survival.

00:01
Primary Sclerosing Cholangitis.
00:04
Break up the name.
00:06
Primarybeginning, arising by
itself.
00:10
Sclerosing; narrowing of what?
The bile duct. That is your topic.
00:15
Next.
00:16
What predictors of what we are going
to discuss?It is an autoimmune disease
for the most part.
00:22
Here, it is not AMA positive.
00:25
AMA positivity was found
in Primary Biliary Cirrhosis.
00:30
Here, upon histology, I'd tell you about
the onion skinning of the bile duct.
00:34
And with primary sclerosing cholangitis,If you narrow,one part of the bile duct,May I ask you a question,
physiologically,If you take any tube,such a blood vessel or a duct
or whatever,If you then narrow the distal
end, what happened to the proximal end?Pathologically.
00:55
Dilation.
00:57
So you havenarrowing.
00:59
Dilation, narrowing, dilation.
01:02
Doesn't it sound awfully
a lot maybe perhapsSome of you might be wearing
necklaces,beaded appearance.
01:10
When would you notice a beaded
appearancewith primary sclerosis cholangitisOn an imaging study.
01:17
And we call that a cholengiogram.
01:20
Let's begin.
01:21
Inflammatory destruction of medium
and large bile ducts;this affects however, young men.
01:27
More so.
01:29
70% are associated with
ulcerated cholitis.
01:31
This is something that you will
never findwith primary biliary cirrhosis.
01:36
you will not find this.
01:37
70% percent is associated
with ulcerated cholitis.
01:41
Where is that?Rectum. Alright?Remember, the rectum,
continuous, ulcers, proximalyso, you move from the rectumup the descending colon
and so forth.
01:54
And because of this, there is an
increased risk of colorectal cancerCRC.
01:59
You see how different PSC is
from PBC.
02:03
Spend a little time to make sure
you are clear between the two diagnoses.
02:08
You cannot afford to get the two confused.
02:11
Along with colorectal cancer,
and ulcerated colitis,there is a major major,association with cholengio carcinoma.
02:19
So, not only there could be cancer
of the colon that mightpredisposed but my goodness,
there is also predispositionof developing cancer of the
gall bladder.
02:28
cholangio carcinoma.
02:30
You won't find this on
Primary biliary cirrhosis.
02:33
Completely different.
02:36
Signs and symptoms here.
02:38
Let me ask you something.
02:40
If there is sclerosing and narrowing
of the bile duct,after the liver,then what then happens?Well, from the liver, you conjugate
bilirubinBut you can't get pastthe bile duct because why?
It is sclerosing.
02:57
So now, if you can't get past
the bile duct into thegall bladder then,you're kind of backing up
don't you?And if you back up enough,
what is that called?We call this post hepatic jaundice.
03:12
Post hepatic jaundice.
Let me ask you something else.
03:15
Did you poop this morning?You probably did.
03:18
If not maybe, whatever,
that is your problem.
03:19
but anyhow, point being is
so you went and passed stoolthis morning maybe.
03:24
And what color was your stool?Did you just take a look at it
at the toilet?Hopefully it was brownish.
right pigmented.
03:30
What contributes the pigmentation
of that stool?Oh the bilirubin.
03:35
Right? bilirubin.
03:37
Not only does it give pigment
to the stool,but it also gives you pigmentto the urine.
03:42
If you can't past that block,in the bile duct.
You can't get the bilirubin,into your intestine.
03:51
If you can't get the bilirubin to
your intestine,Now, what color does your stool look
like?Pale.
03:58
clay.
04:00
Right? Post hepatic jaundice.
04:02
Obstruction. I can't move my
bilirubin further.
04:05
A post hepatic jaundice.
In addition,you have pruritus-itching.
04:11
itching and yellowing of
the skin, you should be thinking, hmm...
04:16
What system are you dealing with?At least know
that you are dealingwith the hepatic-biliary system.
04:20
and fatigue.
04:24
Diagnosis.
04:27
Do an MRCP or ERCP.
04:27
Endoscopic Retrograde Cholangio-PancreatographyThis is then going to give
an imagingwhat is this imaging going
to give you?Remember, I told you, there will
be narrowing or dilationnarrowing, dilation, look for the description
if they don't give you the image.
04:42
the description is calleda beaded appearance.
04:45
If you then take the sclerosed area,and you do a cut section
histology, what are you going to find?hyperplastic changes.
04:53
looks like onion-skinning.
04:55
Any time there is hyperplasia
with a tube, it is called onion-skinning.
04:59
Ok, so don't get hanged up.
There is no such thing as a buzzword.
05:02
I don't really like telling
you that it is onion-skinning.
05:05
Because you know students
immediately think:"Oh, it must be malignant
hypertension Doctor Raj."No, it doesn't have to be.
05:11
Just because I said it is onion-skinning,
I didn't tell you what kind of tube.
05:14
Here, I am telling you it
is a bile duct.
05:17
There is no really effective
therapy.
05:20
Maybe perhaps ursodiol.
05:22
Unfortunately,if you will allow for
the disease to progress,then you're only option at this
point once again will be,liver transplantation.
05:33
You'll notice here, on your left,is PSC - Primary Sclerosing Cholangitis.
05:39
What's shown here is extremely
opaque, it is going to beopacity but then, I want you to go
and start moving down to the bottom.
05:46
And you will notice upon closer examination,a beaded appearance.
05:52
Whereas when you take a look
at the right,if your patient issuffering from gall stones,The most common gallstone
will be your cholesterol stones right?Cholesterol stonesand your cholesterol stones will
be different, that FFFFmeans to say, fatfemale, forty, fertile. Those are
cholesterol stones.
06:14
There is every possibility
that you might accumulatebilirubin if at all your
going to develop gall stones.
06:21
Then it will be a bilirubin
type and it will be pigmented.
06:25
Imaging studies fromERCP- Endoscopic Retrograde Cholangio-PancreatographyBeaded appearance.

USMLE™ is a joint program of the Federation of State Medical Boards
(FSMB®) and National Board of Medical Examiners (NBME®). MCAT is a registered
trademark of the Association of American Medical Colleges (AAMC).
None of the trademark holders are endorsed by nor affiliated with Lecturio.